Document Type

Article

Publication Date

11-2025

Identifier

DOI: 10.1016/j.jhlto.2025.100366; PMCID: PMC12418869

Abstract

The Fontan operation has transformed survival for patients with single ventricle congenital heart disease, but the long-term durability of this physiology remains limited. Fontan circulatory failure (FCF) is a progressive, heterogeneous condition associated with multiorgan dysfunction, lymphatic failure, and elevated post-transplant risk when not recognized early. With increasing survival into adulthood, heart transplantation has emerged as the definitive "fourth stage" of palliation. However, referrals for advanced therapies are often delayed, with many patients presenting in advanced stages of decline. This review synthesizes current evidence on the pathophysiology of Fontan failure, outlines consensus indications for transplant evaluation, and highlights pre-transplant strategies including management of end-organ dysfunction, collateral embolization, and psychosocial readiness that are essential to optimizing outcomes. Advances in surgical technique and mechanical support has markedly improved post-transplant survival, yet challenges remain in timely referral, equitable access, and transition care. Early recognition and multidisciplinary coordination is key to improving outcomes in this vulnerable and growing population.

Journal Title

JHLT Open

Volume

10

First Page

100366

Last Page

100366

PubMed ID

40933271

Keywords

Congenital heart disease; Fontan failure; Fontan-associated liver disease; Heart transplantation; Lymphatic complications; Mechanical circulatory support; Single ventricle; Ventricular assist device

Comments

This article is available under the Creative Commons CC-BY-NC-ND license and permits non-commercial use of the work as published, without adaptation or alteration provided the work is fully attributed.

Publisher's Link: https://www.jhltopen.org/article/S2950-1334(25)00161-2/fulltext

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